THEME: Hope Through Progress: Advancing Sickle
Cell Care Globally
TAGLINE: Screen, Test, Prevent:Writing a Better Story for Sickle Cell🩸 in Nigeria


What is Sickle Cell ?
Sickle Cell Disease (SCD) is a genetic disorder that affects hemoglobin, a protein in red blood cells that carries oxygen to the body's tissues. It is characterized by the production of abnormal hemoglobin, which causes red blood cells to be sickle-shaped, rigid, and prone to clotting.

Prevalence:
SCD is most common among people of African, Mediterranean, Middle Eastern, and South Asian descent. According to the World Health Organization (WHO), SCD affects approximately 4.4% of the global population, with the highest prevalence in Africa, where it affects up to 2% of the population.

Where is it found and who does it affect?
SCD is found globally, but it is most prevalent in Africa, where it affects an estimated 20 million people. It affects people of all ages, genders, and socioeconomic backgrounds.

Facts:
Sickle cell disease is a lifelong problem that affects red blood cells.
- SCD is the most common genetic disorder in Africa.
- SCD affects approximately 1 in 500 births in Nigeria.
- SCD can lead to complications like anemia, infections, and organ damage.
- It is caused by genes that are passed down from parents.
- The disease causes some red blood cells to be shaped like sickles or crescents, which can block blood flow to the rest of the body.
- Sickle cell disease affects more than 100,000 people in the United States and 20 million people worldwide.
- Until recently, a bone marrow transplant was the only cure for sickle cell disease, but in December 2023, the U.S. Food and Drug Administration approved two new therapies to treat the disease.

Myths and Truths:

Myths:

- SCD is contagious.
- SCD is a curse.
- SCD is only found in Africans.
- SCD is only found in Africans. (Truth: SCD affects people of many ethnicities, including Africans, Mediterraneans, Middle Easterners, and South Asians.)
- Sickle cell disease is contagious. (Truth: SCD is a genetic disorder and cannot be caught from someone else.)
- People with sickle cell disease are weak and fragile. (Truth: People with SCD are just as strong and capable as anyone else.)
- Sickle cell disease only affects children. (Truth: SCD affects people of all ages.)
- There is no cure for sickle cell disease. (Truth: While there is no cure, there are treatments and management options available.)

Truths:

- SCD is a genetic disorder that affects hemoglobin production.
- SCD can cause red blood cells to become sickle-shaped, leading to complications.
- SCD can increase the risk of infections, organ damage, and stroke.
- Early diagnosis and treatment can improve quality of life for people with SCD.
- Bone marrow transplants can cure SCD in some cases.
- Genetic counseling and testing can help identify carriers of the sickle cell gene.
- Prenatal testing can detect SCD in unborn babies.
- SCD affects both men and women equally.
- SCD can be managed with blood transfusions, medications, and lifestyle changes.

Where is it found and who does it affect?
SCD is found globally, but it is most prevalent in Africa, where it affects an estimated 20 million people. It affects people of all ages, genders, and socioeconomic backgrounds.


"Screen, Test, Prevent: Let's Write a New Story for Sickle Cell in Nigeria"

Nigeria's Quest for a SCD-Free Generation:

- Improve access to genetic testing and screening.
- Enhance healthcare infrastructure and services for SCD management.
- Increase awareness and education about SCD.
- Support research and development of new treatments and therapies.

High-Risk Gene Combinations:

- SS x SS
- SS x AS
- AS x AS
- SS x AA
- AS x AA

First Aid Treatment:

- Hydration
- Pain management
- Rest
- Oxygen therapy

Screening for Sickle Cell Anemia:

- Newborn screening: a heel prick blood test is done to check for the sickle cell gene
- Genetic screening: a blood or saliva test can detect the sickle cell gene in adults and children
- Family screening: if there is a family history of sickle cell anemia, relatives can be screened to see if they carry the gene

What to Screen For:

- The sickle cell gene (HbS)
- Hemoglobin levels
- Red blood cell count
- Iron levels

Testing for Sickle Cell Anemia:

- Hemoglobin electrophoresis: a lab test that measures the different types of hemoglobin in the blood
- Complete Blood Count (CBC): a test that measures the different components of the blood, including red and white blood cell count
- Sickle Cell Test: a test that detects the presence of sickle-shaped red blood cells
- Genetic testing: a test that detects the sickle cell gene

*What to Test For:*

- Presence of the sickle cell gene (HbS)
- Levels of hemoglobin S (HbS)
- Levels of hemoglobin A (HbA)
- Presence of sickle-shaped red blood cells

*Prevention:*

- Genetic counseling: for families with a history of sickle cell anemia
- Prenatal testing: for pregnant women who are carriers of the sickle cell gene
- Avoiding excessive physical activity
- Staying hydrated
- Avoiding stress
- Getting regular check-ups
Here's a summary:

Unmarried couples can take steps to prevent and prepare for sickle cell disease (SCD):

- Get tested for the sickle cell gene
- Understand test results and the risks of passing the gene to offspring
- Have open conversations about results and risks
- Consider genetic counseling
- Plan carefully if having children, including prenatal testing or preimplantation genetic diagnosis PGD options

By being informed and proactive, couples can make informed decisions about their future together.

Treatment:
Treatment for sickle cell disease (SCD) aims to manage symptoms, prevent complications, and improve quality of life. Some treatments include:

1. Pain management: Medications to alleviate pain episodes.
2. Blood transfusions: Regular transfusions to increase healthy red blood cells.
3. Antibiotics: To treat infections.
4. Folic acid supplements: To help produce healthy red blood cells.
5. Iron chelation therapy: To remove excess iron from the body.
6. Hydroxyurea: A medication to increase fetal hemoglobin production.
7. Stem cell transplant: A potentially curative option.
8. Gene therapy: Experimental treatments to correct genetic defects.
9. Lifestyle modifications: Staying hydrated, avoiding stress, and getting regular exercise.

Newer treatments approved by the FDA include:

1. Adakveo (crizanlizumab): To reduce pain episodes.
2. Oxbryta (voxelotor): To increase hemoglobin production.
3. Zynteglo (betibeglogene autotemcel): A gene therapy for SCD.

It's important to work with a healthcare provider to develop a personalized treatment plan. With proper management, individuals with SCD can lead active and fulfilling lives.
Casgevy which uses CRISPR/Cas9 technology to edit patient's hematopoietic stem cells and increase production of fetal hemoglobin(HbF)

And Lyfgenia which utilizes a lentiviral vector system that modifies the patient's stem cells to produce HbAT87Q, a form of haemoglobin that functions similarly to Hemoglobin A.

Both products are made from patients' own blood and modified and transplanted as a one-time single dose infusion.


Aliyu Usman Bello writes for PANS-BUK Public health team.